Summary:
A 2-year-old-boy presented
to an outside hospital with yellowish discoloration of the right eyelid for 1
week and worsening swelling, redness and ecchymosis of the lid, and periorbital
region for 2 days prior to admission. A computed tomography (CT) scan of the
face and orbit was performed at the outside hospital, which demonstrated a
destructive orbital lesion causing proptosis and periorbital swelling (Figure
1). The patient was transferred to our hospital for further evaluation.
Diagnosis
Adrenal neuroblastoma metastatic to sphenoid wing,
producing “raccoon eye” clinically.
Findings
A CT examination was
performed with a Somatom Definition AS 64 scanner (Siemens, Erlangen, Germany).
The CT scan of the chest, abdomen, and pelvis demonstrate a well-defined
right suprarenal mass with punctate calcifications (Figures 2 and 3).
Discussion
Neuroblastoma is the most common extracranial solid
tumor of childhood, accounting for 8% to 10% of all childhood cancers.1,2
Most children with neuroblastoma are between 1 and 5 years of age, with a
median age at diagnosis of 19 months. Approximately 40% of patients are
infants; 90% are under 5 years of age; and 98% are under 10 years of age.1,3
Neuroblastoma can arise anywhere along the sympathetic chain, from the neck to
the pelvis. Approximately two-thirds occur in the abdomen and 50% to 75% of
these arise in the adrenal medulla. The remaining abdominal tumors arise from
sympathetic paraspinal ganglia. Less common sites of origin are the posterior
mediastinum (10% to 15%), neck (5%), and pelvis (5%).1,2
The common clinical presentation of abdominal
neuroblastoma is an abdominal mass.1,3 Other findings include:
proptosis, periorbital ecchymosis, and bone pain from skeletal metastases,
hepatomegaly from liver metastases, and paraplegia from neural foraminal invasion
and nerve compression.1 Periorbital ecchymoses due to skeletal
metastases frequently produce a clinical appearance called “raccoon eyes.” Raccoon
eyes are also associated with trauma to the orbits and also basilar skull
fractures.4 The raccoon eye in metastatic neuroblastoma is believed
to be due to obstruction of the palpebral veins, which are branches of the
facial and ophthalmic veins.
The spectrum of neuroblastic tumors includes
neuroblastoma, ganglioneuroblastoma, and ganglioneuroma. The tumors decrease in
malignant potential from neuroblastoma to ganglioneuroma with increasing
Schwannian stroma and decreasing malignant neuroblasts. Macroscopically,
neuroblastomas are large masses (mean diameter, 6-8 cm). Areas of hemorrhage,
necrosis and calcification are common on cut section. Approximately 90% of
tumors secrete catecholamines, vanillylmandelic acid (VMA), and homovanillic
acid (HVA).
CT is useful to determine the site and extent of the
tumor and presence of metastatic disease.3,5 Neuroblastoma appears
as a suprarenal or paraspinal mass. The tumor may be homogeneous, but more
often it is heterogeneous, containing calcifications or focal area of necrosis,
hemorrhage, or cystic degeneration. The tumor enhances less than that of
surrounding tissues after administration of intravenous contrast material. Calcifications
are seen in approximately 85% of abdominal neuroblastomas. Neuroblastoma
metastasizes to liver, distant nodes, cortical bone, and bone marrow. Lung
metastases are rare. Skeletal metastases occur in 50% to 60% of patients at
diagnosis, mostly in patients >1 year of age. These can involve cortical
bone or bone marrow. CT findings of metastases include destructive or blastic
lesion, periosteal new bone formation, and a soft-tissue mass.
This patient underwent resection of the adrenal tumor.
Pathology showed numerous immature and mature ganglion cells, consistent with
neuroblastoma with ganglioneuroblastoma elements and was started on a course of
cyclophosphamide and topotecan.
Conclusion
We present a case of an
infant with an adrenal neuroblastoma presenting clinically with periorbital
ecchymoses. The rapidly performed multidetector CT scan allowed diagnosis of a
right suprarenal mass and excellent characterization of the anatomy and extent
of the tumor with exceptionally low radiation levels.
- Pizzo PA, Poplack DG. Neuroblastoma. In: Principles and Practice of Pediatric
Oncology. Philadelphia, Pa: Lippincott Williams & Wilkin; 2011;886-922.
- Gurney JG, Davis S, Severson RK et al. Trends in cancer
incidence among children in the U.S. Cancer. 1996;78:532-541.
- Lonergan GJ, Schwab CM, Suarez ES, Carlson CL.
Neuroblastoma, ganglioneuroblastoma, and ganglioneuroma: Radiologic-pathologic
correlation. Radiographics. 2002;22:911-934.
- Moran
DE, Donoghue V. Periorbital ecchymosis (‘raccoon eyes’) as the presenting
feature of neuroblastoma. PediatrRadiol. 2007;40:1710.
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Babyn P, Siegel MJ.
Adrenal glands, pancreas and other retroperitoneal structures. In: Siegel MJ,
ed. Pediatric Body CT, 2nd ed.
Philadelphia, Pa. Lippincott Williams & Wilkins; 2008;323-360.